Tolosa-Hunt syndrome (THS) is a rare neurological disorder characterized by painful ophthalmoplegia due to idiopathic granulomatous inflammation of the cavernous sinus or orbital apex. This review aims to summarize recent advances in the etiology, diagnosis, and management of THS.

In Guillain-Barré syndrome (GBS) and its variant, anti-GQ1b antibody has a pathogenic role for ophthalmoplegia. In addition, anti-GT1a antibody is related with lower cranial nerve involvement. This report describes a 60-year-old male patient with GBS manifesting with initially isolated dysphagia and subsequently developed ophthalmoplegia. Both immunoglobulin G type anti-GQ1b and anti-GT1a antibodies were detected in the patient’s serum. A mechanism regarding subsequent involvement of respective cranial nerves remains to be elucidated.

Citations

• Gross and Micro-Anatomical Study of the Cavernous Segment of the Abducens Nerve and Its Relationships to Internal Carotid Plexus: Application to Skull Base Surgery
  Grzegorz Wysiadecki, Maciej Radek, R. Shane Tubbs, Joe Iwanaga, Jerzy Walocha, Piotr Brzeziński, Michał Polguj
  Brain Sciences.2021; 11(5): 649.     CrossRef

Acute ophthalmoparesis that includes the oculomotor, trochlear, or abducens nerve may occur as an initial presentation of Miller Fisher syndrome (MFS). The symptoms of MFS or variant forms of Guillain-Barre syndrome are pathogenically related to anti-GQ1b antibodies. We report a case of a 36-year-old man with unilateral isolated abducens nerve palsy associated with anti-GM1 antibody. To the best of our knowledge, this is the first report of unilateral isolated abducens nerve palsy with positivity for anti-GM1 immunoglobulin M antibody.

Citations

• Ganglioside (GM1) Immunoglobulin G Antibody Chronic Bilateral Ophthalmoplegia
  Jesse Cheung, Saif Aldeen Alryalat, Osama Al Deyabat, Andrew G. Lee
  Journal of Neuro-Ophthalmology.2025; 45(1): e31.     CrossRef
• Isolated facial diplegia variant of Guillain–Barré syndrome with anti-GM1 IgG antibody
  Jin Ho Jung, Sukyoon Lee, Jung Hwa Seo, Jong Seok Bae, Kyong Jin Shin, Jong Kuk Kim, Byeol-A Yoon, Seong-il Oh
  Annals of Clinical Neurophysiology.2022; 24(1): 17.     CrossRef

Idiopathic hypertrophic cranial pachymeningitis (ICHP) is diffuse inflammatory process of the dura mater. ICHP can produce similar presentation with Tolosa-Hunt syndrome (THS) if it involves cavernous sinus. A-29-year old male with persistent headache and no definite neurologic dysfunction was noted. Two weeks later, he complained of ophthalmoplegia, and his symptoms were thought to be manifestations of THS. Brain magnetic resonance images revealed diffuse thickened, enhanced pachymeninges in left tentorium. The patient was diagnosed with IHCP. We report a IHCP patient who showed very similar presentation as THS.

Miller-Fisher syndrome, Guillain-Barre syndrome with ophthalmoplegia, Bickerstaff s brainstem encephalitis and acute ophthalmoplegia share some clinical features, and common anti-GQ1b IgG antibody and these are introduced as anti-GQ1b antibody syndrome. These syndromes mostly present with paralysis of extraocular muscles and internal ophthalmoplegia rarely occurs. We report a case of acute isolated bilateral internal ophthalmoplegia associated with anti-GQ1b IgG antibody.

Many neurologic signs are found in Miller Fisher syndrome (MFS) especially including pupillary abnormalities. But when internal ophthalmoparesis is first manifestation in MFS, diagnosis may be difficult in acute phase of illness. We report two cases of MFS presenting with internal ophthalmoplegia. Pupillary areflexia may be involved in acute phase of MFS. When acute bilateral internal ophthalmoparesis is encounted in clinical practice, initial manifestation of MFS should be included in differential diagnosis.

Neuro-ophthalmological findings are common and occasionally prominent features in movement disorders. Accordingly,careful evaluation of the ocular motor functions may provide valuable information in early detection of the diseases andmonitoring of the progression. Furthermore, accurate assessment of the abnormal ocular motor findings aids in understandingthe pathophysiology and mechanisms of the movement disorders, and in their differential diagnosis. Ocular motility examinationshould include bedside evaluation and laboratory recording of the fixational abnormalities, saccades, smooth pursuit, thevestibulo-ocular reflex, optokinetic nystagmus, and vergence eye movements. In this review, we will discuss various ocularmotor findings in ataxia and parkinsonian syndromes, and hyperkinetic movement disorders.